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Sp1 and TAFII130 Transcriptional Activity Disrupted in Early Huntington's Disease
Anthone W. Dunah,1Hyunkyung Jeong,1April Griffin,1Yong-Man Kim,2David G. Standaert,1Steven M. Hersch,1M. Maral Mouradian,2Anne B. Young,1Naoko Tanese,3Dimitri Krainc1*
Huntington's disease (HD) is an inherited
neurodegenerative disease caused by expansion of a polyglutamine tract
in the huntingtinprotein. Transcriptional dysregulation has been
implicated inHD pathogenesis. Here, we report that huntingtin
interacts withthe transcriptional activator Sp1 and coactivator
TAFII130. Coexpressionof Sp1 and TAFII130 in cultured striatal cells
from wild-typeand HD transgenic mice reverses the transcriptional
inhibitionof the dopamine D2 receptor gene caused by mutant
huntingtin,as well as protects neurons from huntingtin-induced
cellular toxicity.Furthermore, soluble mutant huntingtin inhibits Sp1
binding toDNA in postmortem brain tissues of both presymptomatic and
affectedHD patients. Understanding these early molecular events in HDmay provide an opportunity to interfere with the effects of mutanthuntingtin before the development of disease symptoms.
1 Department of Neurology, Massachusetts
General Hospital, Harvard Medical School, Center for Aging, Genetics
and Neurodegeneration, Charlestown, MA 02129, USA.
2 Genetic Pharmacology Unit, Experimental
Therapeutics Branch, National Institute of Neurological Disorders and
Stroke, National Institutes of Health, 10 Center Drive, MSC 1406, Bethesda, MD 20892-1406, USA.
3 Department of
Microbiology/MSB258, New York University School of Medicine, 550 First
Avenue, New York, NY 10016, USA.
*
To whom correspondence should be addressed. E-mail:
krainc{at}helix.mgh.harvard.edu
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